Proton pump inhibitors use suppresses iron absorption in congenital dyserythropoietic anemia.

Congenital dyserythropoietic anemia type I (CDA I) is associated, as other anemic noninflammatory states, with ineffective erythropoiesis and increased iron absorption, which may lead to complication of iron overload. The latter complication requires iron-chelating therapy, which may be associated with adverse effects and toxicity. Gastric acid production is known to be an important factor that facilitates non-heme iron absorption. The purpose of this study was to examine whether treatment with proton pump inhibitors (PPIs) can decrease iron absorption in patients with CDA I. Eight CDA I patients (4 boys) aged 12-18years with mild iron overload (not yet requiring chelating therapy) received 20mg/d omeprazole for 6months. Blood samples were obtained for ferritin, C-reactive protein, hemoglobin, calcium, and magnesium at baseline, at the end of months intervention and 6months after its cessation. The mean ferritin level decreased from 585 +/- 180ng/ml at baseline to 522 +/- 172ng/ml at the end of 6-month treatment and 660 +/- 256ng/ml 6months after cessation of omeprazole treatment (p = 0.009). Omeprazole treatment caused a nonsignificant reduction in the mean iron level (iron 159 +/- 42, 136 +/- 54,167 +/- 34 mu g/dl, p = 0.302). However, mean hemoglobin level was mildly but significantly reduced (Hg 10.0 +/- 0.8, 9.55 +/- 1.0, 10.4 +/- 10.7g/dl, p = 0.002). No adverse effects were reported. Our investigation suggests that administration of PPI to patients with CDA I may reduce iron absorption and may lower iron overload and the need for chelation therapy.